Primary Immunodeficiency

Overview

Primary immunodeficiency disorders — also called primary immune disorders or primary immunodeficiency — weaken the immune system, allowing infections and other health problems to occur more easily.

Many people with primary immunodeficiency are born missing some of the body's immune defenses or with the immune system not working properly, which leaves them more susceptible to germs that can cause infections.

So far, researchers have identified more than 300 forms of primary immunodeficiency disorders. Some forms are so mild they can go unnoticed until adulthood. Other types are severe enough that they're discovered soon after an affected baby is born.

Treatments can boost the immune system in many types of primary immunodeficiency disorders. Research is ongoing, leading to improved treatments and enhanced quality of life for people with the condition.

Symptoms

One of the most common signs of primary immunodeficiency is having infections that are more frequent, longer lasting or harder to treat than are the infections of someone with a typical immune system. You may also get infections that a person with a healthy immune system likely wouldn't get (opportunistic infections).

Signs and symptoms differ depending on the type of primary immunodeficiency disorder, and they vary from person to person.

Signs and symptoms of primary immunodeficiency can include:

• Frequent and recurrent pneumonia, bronchitis, sinus infections, ear infections, meningitis or skin infections

• Inflammation and infection of internal organs

• Blood disorders, such as low platelet count or anemia

• Digestive problems, such as cramping, loss of appetite, nausea and diarrhea

• Delayed growth and development

• Autoimmune disorders, such as lupus, rheumatoid arthritis or type 1 diabetes

When to see a doctor

If you or your child has frequent, recurrent or severe infections or infections that don't respond to treatments, talk to your health care provider. Early diagnosis and treatment of primary immune deficiencies can prevent infections that can cause long-term problems.

Causes

Many primary immunodeficiency disorders are inherited — passed down from one or both parents. Problems in the genetic code that acts as a blueprint for producing the cells of the body (DNA) cause many of these immune system defects.

There are more than 300 types of primary immunodeficiency disorders, and researchers continue to identify more. They can be broadly classified into six groups based on the part of the immune system that's affected:

• B cell (antibody) deficiencies

• T cell deficiencies

• Combination B and T cell deficiencies

• Defective phagocytes

• Complement deficiencies

• Unknown (idiopathic)

Risk factors

The only known risk factor is having a family history of a primary immune deficiency disorder, which increases your risk of having the condition.

If you have a type of primary immune deficiency disorder, you might want to seek genetic counseling if you plan to have a family.

Complications

Complications caused by a primary immunodeficiency disorder vary depending on what type you have. They can include:

• Recurrent infections

• Autoimmune disorders

• Damage to the heart, lungs, nervous system or digestive tract

• Slowed growth

• Increased risk of cancer

• Death from serious infection

Prevention

Because primary immune disorders are caused by genetic changes, there's no way to prevent them. But when you or your child has a weakened immune system, you can take steps to prevent infections:

• Practice good hygiene. Wash your hands with mild soap after using the toilet and before eating.

• Take care of your teeth. Brush your teeth at least twice a day.

• Eat right. A healthy, balanced diet can help prevent infections.

• Be physically active. Staying fit is important to your overall health. Ask your doctor what activities are appropriate for you.

• Get enough sleep. Try to go to sleep and get up at the same time daily, and get the same number of hours of sleep every night.

• Manage stress. Some studies suggest that stress can hamper your immune system. Keep stress in check with massage, meditation, yoga, biofeedback or hobbies. Find what works for you.

• Avoid exposure. Stay away from people with colds or other infections and avoid crowds.

• Ask your doctor about vaccinations. Find out which ones you should have.

Source: https://www.mayoclinic.org/diseases-conditions/primary-immunodeficiency/symptoms-causes/syc-20376905

Asthma inhalers: Which one's right for you?

Asthma inhalers are hand-held, portable devices that deliver medication to your lungs. A variety of asthma inhalers are available to help control asthma symptoms. Finding the right one and using it correctly can help you get the medication you need to prevent or treat asthma attacks.

To find the best inhaler for you, you need to find a balance between the correct medication and the type of inhaler that suits your needs and your ability to use the inhaler correctly. Training from your doctor or other health care provider is essential for learning to use the device you choose correctly.

Metered dose inhalers

These inhalers consist of a pressurized canister containing medication that fits into a boot-shaped plastic mouthpiece. With most metered dose inhalers, medication is released by pushing the canister into the boot.

Some types of metered dose inhalers release medication automatically when you inhale. A few metered dose inhalers have built-in dose counters so that you know how many doses remain. Some newer devices use wireless technology to help you count doses by allowing you to track them with an app downloaded to your phone.

If your metered dose inhaler doesn't have a counter, you'll need to track the number of doses you've used or purchase a separate electronic dose counter to tell when the inhaler is low on medication.

For some people, such as for children or older adults, using a spacer or valved holding chamber with an inhaler might make it easier to inhale the full dose. A spacer holds medication in a tube between the inhaler and your mouth after it's released. A valved holding chamber is a specialized spacer with a one-way valve to help regulate the flow of medication.

Releasing the medication into the spacer allows you to inhale more slowly, increasing the amount that reaches your lungs. Spacers and holding chambers require a prescription.

Dry powder inhaler

Rather than a chemical propellant to push the medication out of the inhaler, you release the medication in these inhalers by breathing in a deep, fast breath. There are multiple dose devices, which hold up to 200 doses, and single dose devices, which you fill with a capsule before each treatment.

Soft mist inhaler

Soft mist inhalers are propellant-free devices that are slightly larger than conventional metered dose inhalers. These devices release a low-velocity aerosol mist that can be slowly inhaled over a longer period of time than metered dose and dry powder inhalers. Soft mist inhalers can be used with a valved holding chamber or a face mask in children.

Other devices

Some people can't use a standard metered dose inhaler or dry powder inhaler. Other types include:

• Metered dose inhaler with a face mask. Generally used for infants or small children, this type uses a standard metered dose inhaler with a spacer. The face mask, which attaches to the spacer, fits over the nose and mouth to make sure the right dose of medication reaches the lungs.

• Nebulizer. This device turns asthma medication into a fine mist breathed in through a mouthpiece or mask worn over the nose and mouth. A nebulizer is generally used for people who can't use an inhaler, such as infants, young children, people who are very ill or people who need large doses of medication.

Work with your doctor to determine which type of inhaler will work best for you. Have your doctor, pharmacist or other health provider show you how to use it.

Using your inhaler correctly is critical in ensuring you get the correct dose of medication to keep your asthma under control. Talk to your doctor if you're having trouble using your inhaler, or if it seems like you're not getting enough medication.

Replace your inhaler if it has passed its expiration date or it shows that all the doses have been used.

Source: https://www.mayoclinic.org/diseases-conditions/asthma/in-depth/asthma-inhalers/art-20046382

Asbestos Awareness: Risks, Diseases, and Safe Handling Tips

What is Asbestos? 

Asbestos is a mineral with tough fibers that can handle heat, electricity and corrosion. Companies used it in building materials, insulation, fireproofing materials, brakes and more because of these properties.

Building materials contain asbestos because it’s an effective insulator. Asbestos in cloth, paper, cement, plastic and other materials makes them stronger.

**Asbestos has been found in thousands of consumer, industrial and commercial products.

Inhaling or ingesting asbestos causes fibers to become trapped in the body. Over decades, trapped asbestos fibers can cause inflammation, scarring and cancer.

Asbestos exposure is the primary cause of mesothelioma. Asbestos also causes a progressive lung disease called asbestosis.

Asbestos mainly comes from Russia, Kazakhstan and China. The toxic mineral was once mined throughout North America. Most commercial asbestos deposits contain 5% to 6% asbestos. Some deposits, such as the Coalinga deposit in California, contain 50% or more asbestos.

Types of Asbestos

The Asbestos Hazard Emergency Response Act of 1986 legally recognizes six types of asbestos. This law, H.R. 5073, was passed during the 99th Congress and requires warning labels on any friable (or easily crumbled) asbestos-containing materials still in routine maintenance areas of a school building.

**Asbestos comes in many mineral forms. All asbestos types cause mesothelioma.

The six main types of asbestos fall into two categories: amphibole and serpentine. The World Health Organization’s International Agency for Research on Cancer, the U.S. Department of Health and Human Services and the Environmental Protection Agency all classify each type of asbestos as a cancer-causing substance.

Amphibole Asbestos

Amphibole asbestos fibers are straight and jagged. They appear needle-like.

• Actinolite

• Amosite

• Anthophyllite

• Crocidolite

• Tremolite

Data from research studies indicate amphibole asbestos can cause cancer with less amounts of exposure than with other types of asbestos. But all identified forms of asbestos can cause diseases.

Serpentine Asbestos

Serpentine asbestos fibers are curly. Chrysotile, also known as “white asbestos,” is the only form of this type of asbestos.

Asbestos-Related Diseases

Scientific studies link asbestos exposure to several diseases, including cancers. Mesothelioma is a type of malignant cancer directly related to asbestos exposure. Asbestos also causes asbestos-related lung cancer, ovarian cancer and laryngeal cancer.

Other asbestos-related diseases include:

• Asbestosis

• COPD

• Diffuse pleural thickening

• Pleural effusions

• Pleural plaques

• Pleuritis

Asbestos causes both benign and malignant diseases. While some asbestos-related diseases are classified as benign, they may have serious impacts on quality of life and can still be life-threatening.

Tips for Safely Handling Asbestos

Some jurisdictions allow homeowners to remove asbestos on their own. Follow these precautions if you are considering DIY asbestos abatement:

• Seal off the work area with plastic sheets and turn off the air conditioning or furnace.

• Wear a respirator with a high-efficiency particulate air (HEPA) filter.

• Wear disposable coveralls and gloves during asbestos work.

• Use a pump sprayer at all times to keep asbestos materials wet and suppress dust.

• Clean the work area with wet wipes or a vacuum cleaner with a HEPA filter.

• Dispose of asbestos waste in clearly labeled bags at a landfill that can accept asbestos.

In many situations that require the removal of asbestos-containing materials, it may be safer to leave the materials undisturbed or encapsulate them with a sealant. Consult a certified, local asbestos abatement professional for the best advice.

source: https://www.asbestos.com/asbestos/#what-is-asbestos